Association of Peripheral Blood and Cutaneous Eosinophils With Bullous Pemphigoid Disease Severity and Treatment Outcomes / Asociación entre eosinófilos de sangre periférica y cutáneos con la gravedad del penfigoide ampolloso y los resultados terapéuticos

Background and aims A dermal inflammatory infiltrate rich in eosinophils is a prominent histological feature of bullous pemphigoid (BP) and peripheral blood eosinophilia has been documented in 50–60% of BP patients. Nevertheless, the impact of circulating and dermal infiltrate eosinophil levels on BP remains poorly understood. The main objective of this work was to investigate the association of peripheral blood and dermal infiltrate eosinophil levels with clinical and immunological characteristics of the disease. Material and methods Retrospective cohort study including all patients diagnosed with BP between 2011 and 2020. Results The study cohort included 233 patients with BP. The mean baseline peripheral blood eosinophil count was 956.3±408.6×106/L and the mean number of tissue eosinophils at the dermal hot spot area was 30.5±19.0. Patients with disseminated presentation (i.e. BSA>50%) had significantly higher peripheral blood eosinophil counts (P=0.028). Mucosal involvement was significantly associated with lower dermal eosinophil count (P=0.001). Requiring inpatient care and relapsing were significantly associated with high peripheral blood eosinophil count (P=0.025; P=0.020, respectively). Among the 68 patients who experienced a relapse, 31 had peripheral blood eosinophilia (i.e. >500×106/L) at relapse (44.2%). Peripheral blood eosinophil counts at baseline and at relapse were significantly correlated (r=0.82, P<0.001). Conclusion Peripheral blood and cutaneous eosinophils levels may be useful biomarkers for disease activity and treatment outcomes in BP. Monitoring peripheral blood eosinophil counts may allow early detection of relapse (AU)

Antecedentes y objetivos El infiltrado inflamatorio dérmico rico en eosinófilos es una característica histológica destacada de penfigoide ampolloso (PA) y eosinofilia en sangre periférica, que se ha documentado en el 50-60% de los pacientes con esta enfermedad. Sin embargo, el impacto de los niveles de eosinófilos circulantes y en infiltrados dérmicos en el PA sigue sin comprenderse. El objetivo principal de este estudio fue investigar la asociación entre los niveles de eosinófilos en sangre periférica y en infiltrados dérmicos y las características clínicas e inmunológicas de esta enfermedad. Material y métodos Estudio de cohorte retrospectivo que incluyó a todos los pacientes con PA entre 2011 y 2020. Resultados El estudio de cohorte incluyó 233 pacientes con PA. El recuento de eosinófilos basal medio en sangre periférica fue de 956,3 ± 408,6 x106/L y el número medio de eosinófilos tisulares en la zona dérmica clave fue de 30,5 ± 19. Los pacientes con presentación diseminada (es decir, BSA >50%) tuvieron conteos de eosinófilos en sangre periférica significativamente superiores (p = 0,028). El compromiso mucoso estuvo significativamente asociado a un conteo de eosinófilos cutáneo inferior (p = 0,001). La necesidad de cuidados hospitalarios y las recaídas estuvieron significativamente asociadas a conteos de eosinófilos en sangre periférica más elevados (p = 0,025; p = 0,020, respectivamente). Entre los 68 pacientes que experimentaron recidiva, 31 tuvieron eosinofilia en sangre periférica (es decir, > 500 x 106/L) en recaída (44,2%). Los conteos de eosinófilos en sangre periférica basales y en recaída se correlacionaron significativamente (r = 0,82, p < 0,001). Conclusione Los niveles de eosinófilos en sangre periférica y cutáneos pueden constituir biomarcadores útiles para la actividad de la enfermedad y los resultados terapéuticos en el PA. Supervisar los conteos de eosinófilos en sangre periférica puede ayudar a detectar la recidiva tempranamente (AU)

[Artículo traducido] Asociación entre eosinófilos de sangre periférica y cutáneos con la gravedad del penfigoide ampolloso y los resultados terapéuticos / Association of Peripheral Blood and Cutaneous Eosinophils With Bullous Pemphigoid Disease Severity and Treatment Outcomes

Antecedentes y objetivos El infiltrado inflamatorio dérmico rico en eosinófilos es una característica histológica destacada de penfigoide ampolloso (PA) y eosinofilia en sangre periférica, que se ha documentado en el 50-60% de los pacientes con esta enfermedad. Sin embargo, el impacto de los niveles de eosinófilos circulantes y en infiltrados dérmicos en el PA sigue sin comprenderse. El objetivo principal de este estudio fue investigar la asociación entre los niveles de eosinófilos en sangre periférica y en infiltrados dérmicos y las características clínicas e inmunológicas de esta enfermedad. Material y métodos Estudio de cohorte retrospectivo que incluyó a todos los pacientes con PA entre 2011 y 2020. Resultados El estudio de cohorte incluyó 233 pacientes con PA. El recuento de eosinófilos basal medio en sangre periférica fue de 956,3 ± 408,6 x106/L y el número medio de eosinófilos tisulares en la zona dérmica clave fue de 30,5 ± 19. Los pacientes con presentación diseminada (es decir, BSA >50%) tuvieron conteos de eosinófilos en sangre periférica significativamente superiores (p = 0,028). El compromiso mucoso estuvo significativamente asociado a un conteo de eosinófilos cutáneo inferior (p = 0,001). La necesidad de cuidados hospitalarios y las recaídas estuvieron significativamente asociadas a conteos de eosinófilos en sangre periférica más elevados (p = 0,025; p = 0,020, respectivamente). Entre los 68 pacientes que experimentaron recidiva, 31 tuvieron eosinofilia en sangre periférica (es decir, > 500 x 106/L) en recaída (44,2%). Los conteos de eosinófilos en sangre periférica basales y en recaída se correlacionaron significativamente (r = 0,82, p < 0,001). Conclusione Los niveles de eosinófilos en sangre periférica y cutáneos pueden constituir biomarcadores útiles para la actividad de la enfermedad y los resultados terapéuticos en el PA. Supervisar los conteos de eosinófilos en sangre periférica puede ayudar a detectar la recidiva tempranamente (AU)

Background and aims A dermal inflammatory infiltrate rich in eosinophils is a prominent histological feature of bullous pemphigoid (BP) and peripheral blood eosinophilia has been documented in 50–60% of BP patients. Nevertheless, the impact of circulating and dermal infiltrate eosinophil levels on BP remains poorly understood. The main objective of this work was to investigate the association of peripheral blood and dermal infiltrate eosinophil levels with clinical and immunological characteristics of the disease. Material and methods Retrospective cohort study including all patients diagnosed with BP between 2011 and 2020. Results The study cohort included 233 patients with BP. The mean baseline peripheral blood eosinophil count was 956.3±408.6×106/L and the mean number of tissue eosinophils at the dermal hot spot area was 30.5±19.0. Patients with disseminated presentation (i.e. BSA>50%) had significantly higher peripheral blood eosinophil counts (P=0.028). Mucosal involvement was significantly associated with lower dermal eosinophil count (P=0.001). Requiring inpatient care and relapsing were significantly associated with high peripheral blood eosinophil count (P=0.025; P=0.020, respectively). Among the 68 patients who experienced a relapse, 31 had peripheral blood eosinophilia (i.e. >500×106/L) at relapse (44.2%). Peripheral blood eosinophil counts at baseline and at relapse were significantly correlated (r=0.82, P<0.001). Conclusion Peripheral blood and cutaneous eosinophils levels may be useful biomarkers for disease activity and treatment outcomes in BP. Monitoring peripheral blood eosinophil counts may allow early detection of relapse (AU)

Association of Peripheral Blood and Cutaneous Eosinophils With Bullous Pemphigoid Disease Severity and Treatment Outcomes.

BACKGROUND AND AIMS: A dermal inflammatory infiltrate rich in eosinophils is a prominent histological feature of bullous pemphigoid (BP) and peripheral blood eosinophilia has been documented in 50-60% of BP patients. Nevertheless, the impact of circulating and dermal infiltrate eosinophil levels on BP remains poorly understood. The main objective of this work was to investigate the association of peripheral blood and dermal infiltrate eosinophil levels with clinical and immunological characteristics of the disease. MATERIAL AND METHODS: Retrospective cohort study including all patients diagnosed with BP between 2011 and 2020. RESULTS: The study cohort included 233 patients with BP. The mean baseline peripheral blood eosinophil count was 956.3±408.6×106/L and the mean number of tissue eosinophils at the dermal hot spot area was 30.5±19.0. Patients with disseminated presentation (i.e. BSA>50%) had significantly higher peripheral blood eosinophil counts (P=0.028). Mucosal involvement was significantly associated with lower dermal eosinophil count (P=0.001). Requiring inpatient care and relapsing were significantly associated with high peripheral blood eosinophil count (P=0.025; P=0.020, respectively). Among the 68 patients who experienced a relapse, 31 had peripheral blood eosinophilia (i.e. >500×106/L) at relapse (44.2%). Peripheral blood eosinophil counts at baseline and at relapse were significantly correlated (r=0.82, P<0.001). CONCLUSIONS: Peripheral blood and cutaneous eosinophils levels may be useful biomarkers for disease activity and treatment outcomes in BP. Monitoring peripheral blood eosinophil counts may allow early detection of relapse.

Lesión erosiva y supurativa unilateral del pezón / Unilateral Erosive and Oozing Lesion of the Nipple

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Adalimumab-induced psoriatic alopecia/alopecia areata-like reaction in a patient with Crohn's disease.

Anti-tumor necrosis factor (anti-TNF) agents have been successfully used to treat both chronic idiopathic inflammatory bowel disease and other immune-mediated chronic diseases, but they can also induce a wide array of cutaneous reactions, including new-onset psoriasis and alopecia. We report a case of alopecia associated with psoriasiform skin lesions in a patient on adalimumab treatment for Crohn's disease.

Herpes simplex virus and cytomegalovirus co-infection presenting as exuberant genital ulcer in a woman infected with human immunodeficiency virus.

In patients infected with human immunodeficiency virus (HIV), genital herpes can result in severe and atypical clinical presentations, and can become resistant to aciclovir treatment. Rarely, these manifestations may represent concurrent herpes simplex virus (HSV) with other agents. We report a 41-year-old black woman with HIV who presented with extensive and painful ulceration of the genitalia. Histological examination of a biopsy sample was suggestive of herpetic infection, and intravenous aciclovir was started, but produced only partial improvement. PCR was performed on the biopsy sample, and both HSV and cytomegalovirus (CMV) DNA was detected. Oral valganciclovir was started with therapeutic success. CMV infection is common in patients infected with HIV, but its presence in mucocutaneous lesions is rarely reported. This case exemplifies the difficulties of diagnosis of genital ulcers in patients infected with HIV. The presence of exuberant and persistent HSV genital ulcers in patients with HIV should also raise suspicions of the presence of co-infection with other organisms such as CMV.

An atypical case of cutaneous leishmaniasis caused by Leishmania infantum in Portugal.

Leishmaniasis is a parasitic disease caused by an intracellular protozoan that belongs to the genus Leishmania and is transmitted by a phlebotomine sandfly. In Southwest Europe, including Portugal, cutaneous leishmaniasis is considered a rare disease of unknown or underestimated prevalence. Leishmania infantum is the only species identified as responsible for the autochthonous cases.We report the case of a 66-year-old man with an erythematous, painless plaque on the mid face region, accompanied by nasal obstruction with 9 months of evolution. The initial diagnoses were: lymphoma, subcutaneous mycosis, Wegener's granulomatosis, and lupus vulgaris. The diagnosis of leishmaniasis was based on histopathology findings and identification of L. infantum by DNA based methods. Blood cultures, abdominal ultrasound and myelogram ruled out systemic involvement. The patient was treated with intravenous meglumine antimoniate (20 mg per kg/day) for four weeks, without major side effects.We emphasize the importance of this case because human cutaneous leishmaniasis has rarely been diagnosed in Portugal and some cases are atypical, such as the situation herein described.

[Lipomembranous panniculitis: clinicopathologic correlation of 8 cases]. / Paniculitis lipomembranosa: correlación clínico-patológica de 8 casos.

INTRODUCTION: The term lipomembranous panniculitis refers to a chronic inflammation of the subcutaneous cell tissue, probably representing a non-specific type of ischemic necrosis of the fatty tissue, common to several complaints. It is characterized by painful sclerotic subcutaneous deposits, located in the lower legs of obese, middle-aged woman, with a history of vascular insufficiency and stasis dermatitis. The condition may also appear in association with other inflammatory disorders. METHODS: This paper is a review of all cases of lipomembranous panniculitis diagnosed at the Skin Histopathology Laboratory at Santa Maria Hospital, Lisbon, from 1985-2005. In the histopathological study, the associated clinical processes were retrospectively analyzed and the risk factors/associated pathologies re-evaluated. RESULTS: Eight patients were identified, all women, and with an average age of 49. Over half the patients were overweight. In most cases the lesions had been evolving for over 6 months and were associated with chronic venous insufficiency of the legs, both from a clinical point of view (7 patients) and a histopathological point of view (6 patients). In two of the cases, the lesions occurred in patients with connective tissue disorders. CONCLUSION: Great variability was observed with regard to the clinical morphology of the lesions, the proposed diagnoses and prescribed treatments, all of which possibly highlight the non-specific nature of the process.

Paniculitis lipomembranosa: correlación clínico-patológica de 8 casos / Lipomembranous panniculitis: clinicopathologic correlation of 8 cases

Introducción. El término paniculitis lipomembranosa designa un proceso inflamatorio crónico del tejido celular subcutáneo que probablemente representa una forma inespecífica de necrosis isquémica del tejido adiposo, común a diversas entidades clínicas. Clínicamente se caracteriza por placas subcutáneas escleróticas, dolorosas, localizadas en las áreas distales de las piernas de mujeres obesas, de mediana edad, con una base de insuficiencia vascular y dermatitis de estasis. El proceso puede también desarrollarse en asociación con otras patologías inflamatorias. Métodos. El presente trabajo constituye una revisión de todos los casos de paniculitis lipomembranosa diagnosticados en el Laboratorio de Histopatología Cutánea del Hospital de Santa María de Lisboa entre 1985 y 2005. Se realizó un análisis retrospectivo de los procesos clínicos asociados y una reevaluación de los factores de riesgo/patologías asociadas en el estudio histopatológico. Resultados. Se identificaron 8 pacientes, todos del sexo femenino, con una media de edad de 49 años y más de la mitad con exceso de peso. En la mayoría de los casos las lesiones llevaban más de 6 meses de evolución y estaban asociadas a insuficiencia venosa crónica de las extremidades inferiores, tanto desde el punto de vista clínico (7 pacientes), como histopatológico (6 pacientes). En dos de los casos, las lesiones se presentaron en pacientes con conectivopatías. Conclusión. Se observó una gran variabilidad en cuanto a la morfología clínica de las lesiones, los diagnósticos propuestos y las terapéuticas administradas, lo que probablemente subraya también el carácter inespecífico de este proceso

Introduction. The term lipomembranous panniculitis refers to a chronic inflammation of the subcutaneous cell tissue, probably representing a non-specific type of ischemic necrosis of the fatty tissue, common to several complaints. It is characterized by painful sclerotic subcutaneous deposits, located in the lower legs of obese, middle-aged woman, with a history of vascular insufficiency and stasis dermatitis. The condition may also appear in association with other inflammatory disorders. Methods. This paper is a review of all cases of lipomembranous panniculitis diagnosed at the Skin Histopathology Laboratory at Santa Maria Hospital, Lisbon, from 1985-2005. In the histopathological study, the associated clinical processes were retrospectively analyzed and the risk factors/associated pathologies re-evaluated. Results. Eight patients were identified, all women, and with an average age of 49. Over half the patients were overweight. In most cases the lesions had been evolving for over 6 months and were associated with chronic venous insufficiency of the legs, both from a clinical point of view (7 patients) and a histopathological point of view (6 patients). In two of the cases, the lesions occurred in patients with connective tissue disorders. Conclusion. Great variability was observed with regard to the clinical morphology of the lesions, the proposed diagnoses and prescribed treatments, all of which possibly highlight the non-specific nature of the process

A7445G mtDNA mutation present in a Portuguese family exhibiting hereditary deafness and palmoplantar keratoderma.

Mitochondrial DNA (mtDNA) A7445G point mutation has been shown to be responsible for familial nonepidermolytic palmoplantar keratoderma (NEPPK) associated with deafness without any additional features. To date, only a few cases have been described. We report a Portuguese pedigree presenting an inherited combination of NEPPK and sensorineural deafness compatible with maternal transmission. Clinical expression and age of onset of NEPPK and deafness were variable. Normal expression patterns of epidermal keratins and filaggrin, intercellular junction proteins including connexin 26, loricrin and cornified envelope proteins, were observed. Molecular analysis revealed that all the affected members, previously screened for Cx26 mutations with negative results, presented the mtDNA A7445G point mutation in the homoplasmic form. To our knowledge, this is the fifth family in whom inherited NEPPK and hearing loss are related to this mitochondrial mutation.

Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases.

AIM: To review the dinical and histological data of 20 cases of dermatofibrosarcoma protuberans presenting at two dermatology centres in Lisbon from 1978 to 1998. PATIENTS AND METHODS: The 20 subjects comprised nine males and 11 females ranging in age from 25 to 79 years, with highest frequency of subjects in the 30-50 year olds. We reviewed the clinical features, histopathological aspects, including morphologic variants and immunohistochemical studies. RESULTS: Median age at diagnosis was 51 years and the trunk was the most frequent location. The characteristic histologic storiform pattern was seen in all cases. Three subjects presented fibrosarcomatous areas, one with myoid differentiation and another with multinucleated giant cells. Immunohistochemical stains revealed CD34 expression in the 18 specimens tested, FXIIIa was negative, and these two antigens proved important for the differential diagnosis of this neoplasm. Local wide excision was performed in 13 cases and seven patients underwent Moh's micrographic surgery. Follow-up ranged from 2 months to 17 years and three recurrences were recorded, two following classical surgery and one after Moh's surgery; there was no difference in the rate of local recurrence (15%) for the two kinds of treatment in our series.